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Scoliosis (Idiopathic), Pediatric

Basics

Description

- Hormonal - Lower levels of melatonin secreted from pineal body in those with adolescent idiopathic scoliosis - Growth hormone: more of an influential factor than an etiologic factor in studies - Vertebral growth abnormalities - Asymmetric growth rates between the right and left sides of the spine

- Adolescent idiopathic scoliosis (11 " 17 years) - Juvenile idiopathic scoliosis (4 " 10 years) - Infantile idiopathic scoliosis (0 " 3 years) - Congenital scoliosis " due to bony abnormalities of the spine that are present at birth (failure of formation or segmentations of vertebrae) - Scoliosis associated with neurofibromatosis - Scoliosis associated with tumors (e.g., osteoid osteoma) - Neuromuscular scoliosis (e.g., cerebral palsy, spina bifida, muscle disorders) - Postural scoliosis (e.g., from leg length discrepancy) - No rib hump or rotation - Does not have fixed deformities - Disappears with forward bending - Long curve - No progression

- Curves ≥25 degrees (in Risser 0 " 3 patient) that have demonstrated >10 degrees progression during period of observation - Continue brace treatment until maturity (2 years postmenarchal and Risser 4 in females, Risser 5 in males).

- Brace types - Thoracolumbosacral orthosis (TLSO): success reported when used >16 " 18 hours daily; significantly improved outcome when compared with natural history - Cervicothoracolumbosacral orthosis (CTLSO): seldom needed except for higher thoracic or cervical curves - Nighttime bending brace

  • Scoliosis: lateral curvature of spine exceeding 10 degrees on PA full-spine radiograph (with rotation of spine); curves <10 are termed spinal asymmetry; considered idiopathic only after other causes have been excluded
  • Kyphosis: anteriorly concave curvature of vertebral column

Epidemiology

  • Female-to-male ratios:1.4:1 for curves 11 " 20 degrees5.4:1 for curves >20 degrees

Prevalence

  • Generally considered 1.5 " 3% for curves ≥10 degrees
  • 0.3 " 0.5% for curves >20 degrees

Risk Factors

Genetics

Positive familial history for idiopathic scoliosis in 30% (not predictive of severity)

  • Under active investigation: GWAS and whole exome sequencing studies
  • Several candidate genes have been identified.

Etiology

By definition, unknown; listed are some theories, none proven in isolation:

  • GeneticPositive familial history for scoliosis in 30% (not predictive of severity)
  • Connective tissue disorderAssociated with several connective tissue disorders (including Marfan syndrome, Ehlers-Danlos syndrome, etc.)Alterations in connective tissue of the spine, paraspinous muscles, and plateletsMay be related to osteopenia (decreased bone mineral density) of vertebral bodies
  • Neurologic (equilibrium system)Abnormalities noted in vestibular, ocular, proprioceptive, and vibratory functions
  • HormonalLower levels of melatonin secreted from pineal body in those with adolescent idiopathic scoliosisGrowth hormone: more of an influential factor than an etiologic factor in studiesVertebral growth abnormalitiesAsymmetric growth rates between the right and left sides of the spine

Commonly Associated Conditions

  • Connective tissue disorders, including Marfan syndrome and Ehlers-Danlos syndrome
  • Neurofibromatosis
  • Neuromuscular conditions, including cerebral palsy, spina bifida, spinal muscular atrophy, Friedreich ataxia, etc.
  • If any of these conditions are present, the diagnosis is no longer idiopathic.

Diagnosis

History

  • Onset: Consider when first noted, by whom, rate of worsening, previous treatment, patient recent growth, the physical change of puberty, associated signs or symptoms, familial history, etc.
  • Patients with idiopathic scoliosis usually should not have pain, although they might have a discomfort or mild pain.
  • Back pain in scoliotic patients must be investigated thoroughly and taken seriously.
  • If night pain, consider tumor such as osteoid osteoma.

Physical Exam

  • General inspection to look for skin changes such as cafe au lait spots, pigmentation, or other signs of neurofibromatosis; also dysraphic signs (e.g., hairy patches, midline hemangioma, skin dimpling)
  • Assess for skeletal maturity, hyperelasticity, contracture, congenital anomalies.
  • Assess for deformity; asymmetry of spine, shoulders, waist, and trunk, including decompensation; abnormalities of thoracic kyphosis or cervical or lumbar lordosis; disfigurement of the torso; or rib rotation.
  • Adams forward bend test used to look for rib or paraspinous elevations
  • Assess for leg length discrepancy, congenital anomalies, and neurologic abnormalities (including abnormal abdominal reflex).
  • Special finding:Crankshaft phenomenonProgression of curve size and rotation following posterior spinal fusion in a young child, result of continued anterior spinal growthPatient is Risser 0, open triradiate cartilages, <10 years old, and prior to occurrence of peak height velocity (time of maximum spinal growth).Consider anterior fusion in addition to posterior fusion.
  • Physical exam tricks:Measure angle of trunk rotation with scoliometer.Abnormal abdominal reflex may suggest intraspinal pathology, including syrinx.
  • Perform Adams forward bend test after the pelvis is leveled by inserting appropriately sized block underneath the short leg in patients with scoliosis and leg length discrepancy.

Diagnostic Tests & Interpretation

Pulmonary function testing is useful preoperatively for more severe curves.

Lab

Usually not helpful unless to rule out associated metabolic conditions

Imaging

  • Plain standing posterior " anterior and lateral scoliosis films on long 3-foot radiograph cassette or 3-D low-dose radiation system
  • One must look for soft tissue and congenital bony abnormalities (Wedge vertebrae, bars, hemivertebrae).
  • Curve is measured using Cobb method.
  • The status of the triradiate cartilage and Risser classification of iliac apophysis ossification are indicators of maturity.
  • The triradiate cartilage usually closes before the iliac apophysis appears (Risser 0).
  • Risser sign is defined by the amount of calcification present in the iliac apophysis and measures the progressive ossification from anterolaterally to posteromedially.A Risser grade of 1 signifies up to 25% ossification of the iliac apophysis, proceeding to grade 4, which signifies 100% ossification.A Risser grade of 5 means the iliac apophysis has fused to the iliac crest after 100% ossification.Risser grade (0 " 5) gives an estimate of how much skeletal growth remains and is correlated with risk of curve progression.
  • MRI is not routinely necessary for adult idiopathic scoliosis without back pain.
  • 7% prevalence of intraspinal abnormalities are found in left thoracic curves, so MRI maybe indicated.
  • Curve patterns are classified according to King or Lenke classifications.
  • Renal ultrasound is used for evaluation of patient with congenital scoliosis (look for associated renal abnormalities).

Differential Diagnosis

  • Adolescent idiopathic scoliosis (11 " 17 years)
  • Juvenile idiopathic scoliosis (4 " 10 years)
  • Infantile idiopathic scoliosis (0 " 3 years)
  • Congenital scoliosis " due to bony abnormalities of the spine that are present at birth (failure of formation or segmentations of vertebrae)
  • Scoliosis associated with neurofibromatosis
  • Scoliosis associated with tumors (e.g., osteoid osteoma)
  • Neuromuscular scoliosis (e.g., cerebral palsy, spina bifida, muscle disorders)
  • Postural scoliosis (e.g., from leg length discrepancy)No rib hump or rotationDoes not have fixed deformitiesDisappears with forward bendingLong curveNo progression

Treatment

General Measures

  • TreatmentConcepts for treatment are based on severity of deformity and on likelihood of progression.
  • ObservationCurves <25 degreesImmature patients (Risser 0, 1, 2) should be reevaluated in 4 " 6 months.Skeletally mature patients (Risser 4 or 5) usually do not require ongoing follow-up unless special circumstances exist.Curves 25 " 45 degrees in skeletally mature patientsRisser 4 or 5 patients are usually reevaluated in 6 months to 1 year.Mature patients are usually reevaluated yearly.

Additional Therapies

  • Brace treatmentCurves 25 " 45 degrees (Risser 0, 1) and 30 " 45 degrees (Risser 2 or 3)Brace on initial evaluation.Curves ≥25 degrees (in Risser 0 " 3 patient) that have demonstrated >10 degrees progression during period of observationContinue brace treatment until maturity (2 years postmenarchal and Risser 4 in females, Risser 5 in males).
  • Brace typesThoracolumbosacral orthosis (TLSO): success reported when used >16 " 18 hours daily; significantly improved outcome when compared with natural historyCervicothoracolumbosacral orthosis (CTLSO): seldom needed except for higher thoracic or cervical curvesNighttime bending brace

Surgery/Other Procedures

  • Recommended when curves exceed 45 " 50 degreesException: Balanced thoracic and lumbar curves <55 degrees may be observed for progression.
  • Thoracic curves and double major curvesPosterior segmental fixation instrumentation remains current state of the art.Anterior spinal instrumentation for selected curves
  • Isolated thoracolumbar and lumbar curvesAnterior spinal fusion using solid rod segmental constructs

Ongoing Care

Follow-up Recommendations

Patient Monitoring

  • Watch for back pain associated with idiopathic scoliosis (may indicate other diagnosis):Present in 23% at time of initial evaluation (additional 9% during follow-up)Of those with back pain, only 9% found to have identifiable cause such as spondylolysis, Scheuermann, syrinx, disc herniation, tumor, tether cord.

Prognosis

  • Overall, good for most patients
  • Risk of curve progression related to patient 's maturity (Risser sign, menarcheal status) and to size of curve
  • Curves <20 " 25 degrees have low risk of progression, even if patient is immature.
  • Curves 25 " 45 degrees have higher risk of progression, particularly in skeletally immature patients.
  • Curves >45 " 50 degrees have much higher risk of progression, regardless of maturity.

Complications

Natural history:

  • Reduced pulmonary function for patients with thoracic curves >60 degrees
  • Progression of lumbar curves >50 degrees in adult life with degenerative disc disease and pain in some
  • Cosmetic and emotional issues
  • Complications of brace use include skin irritation, discomfort, and noncompliance.
  • Surgical complications are usually more severe, including infection, instrumentation loosening or breakage, neurologic damage, paralysis, or death.

Additional Reading

  • Dormans JP. Establishing a standard of care for neuromonitoring during spinal deformity surgery. Spine. 2010;35(25):2180 " 2185. [View Abstract]
  • Hresko MT. Clinical practice. Idiopathic scoliosis in adolescents. N Engl J Med. 2013;368(9):834 " 841. [View Abstract]
  • El-Hawary R, Chukwunyerenwa C. Update on evaluation and treatment of scoliosis. Pediatr Clin North Am. 2014;61(6):1223 " 1241.
  • Sponseller PD, Flynn JM, Newton PO, et al. The association of patient characteristics and spinal curve parameters with Lenke classification types. Spine. 2012;37(13):1138 " 1141. [View Abstract]
  • Sucato DJ. Management of severe spinal deformity: scoliosis and kyphosis. Spine. 2010;35(25):2186 " 2192. [View Abstract]

Codes

ICD09

  • 737.30 Scoliosis [and kyphoscoliosis], idiopathic
  • 737.10 Kyphosis (acquired) (postural)
  • 737.20 Lordosis (acquired) (postural)
  • 737.32 Progressive infantile idiopathic scoliosis
  • 737.31 Resolving infantile idiopathic scoliosis
  • 737.9 Unspecified curvature of spine
  • 754.2 Congenital musculoskeletal deformities of spine

ICD10

  • M41.9 Scoliosis, unspecified
  • M40.209 Unspecified kyphosis, site unspecified
  • M40.56 Lordosis, unspecified, lumbar region
  • M41.00 Infantile idiopathic scoliosis, site unspecified
  • M41.129 Adolescent idiopathic scoliosis, site unspecified
  • M40.204 Unspecified kyphosis, thoracic region
  • Q67.5 Congenital deformity of spine
  • M40.50 Lordosis, unspecified, site unspecified
  • M41.20 Other idiopathic scoliosis, site unspecified
  • M41.119 Juvenile idiopathic scoliosis, site unspecified

SNOMED

  • 298382003 Scoliosis deformity of spine (disorder)
  • 405773007 Kyphoscoliosis deformity of spine (disorder)
  • 61960001 Lordosis deformity of spine (disorder)
  • 20980008 Progressive infantile idiopathic scoliosis (disorder)
  • 298493002 Kyphosis deformity of thoracic spine (disorder)
  • 287087003 Congenital lordosis/scoliosis (disorder)
  • 298494008 Scoliosis of thoracic spine (disorder)
  • 28801006 Resolving infantile idiopathic scoliosis (disorder)

FAQ

  • Q: How long do you observe a patient with spinal asymmetry before ordering a radiograph?
  • A: It depends on the presence or absence of abnormalities on the physical exam. If any of the signs mentioned here are seen or significant back pain is present, a radiograph or referral is indicated. The scoliometer is also a useful tool in screening patients.
  • Q: How long do you observe a patient with spinal asymmetry before referral to an orthopedic surgeon?
  • A: Consider referral ifCobb angle>20 degreesProgression of more than 5 degrees
  • Q: If a child presents with scoliosis and back pain that occurs especially at night and is promptly relieved with nonsteroidal anti-inflammatory drugs, what diagnosis is suggested?
  • A: Scoliosis associated with osteoid osteoma.